Cardiac sarcoidosis completely mimicking biventricular arrhythmogenic cardiomyopathy

Abstract

Cardiac sarcoidosis (CS) is a chameleon of cardiology, and it can mimic different cardiac diseases; among them is arrhythmogenic cardiomyopathy (ACM). We admitted a 70-year-old female patient with heart failure symptoms in 2015, who fulfilled all major ECG and non-invasive imaging criteria of biventricular ACM. She was well with the recommended medications for 3 years, showing only isolated cardiac involvement, but in 2018, cervical and mediastinal lymphadenopathy appeared and cervical lymph node core biopsy histology, bronchoalveolar lavage flow cytometry strongly suggested extracardiac sarcoidosis. Therefore, our suspicion was that sarcoidosis is responsible for the cardiac involvement, which was not confirmed by PET-CT and gallium scintigraphy examinations. At the end of 2018, she died in septicaemia with multiorgan failure, and only autopsy verified her CS. A new ECG algorithm published in 2021 for the differential diagnosis of CS and biventricular ACM, when applied on her ECGs recorded in 2015, suggested the diagnosis of CS.

Cardiac Sarcoidosis: A Comprehensive Clinical Review

“…CS may completely mimic ACM with biventricular involvement, which occurs more frequently (in 56% of patients with ACM) than the classic right ventricular (RV) dominant or isolated RV involvement form (in 39%) [ 37 , 38 ], fulfilling all major non-invasive imaging and ECG criteria of ACM. CS can also be associated with T-wave inversion in right precordial leads ( ) in the absence of RBBB, which is a major ECG criterion of ACM and with Ԑ-wave, which earlier was considered an almost pathognomonic ECG sign of ACM, but now is only a minor ECG criterion of ACM [ 6 , 10 , 19 , 38 ]. Recently Hoogendoorn JC et al .…”

“…[ 6 ]) including PR interval of 220 ms, the presence of R’ wave and the surface area of maximum R’ wave in leads 1.65 to distinguish CS with biventricular involvement from ACM with biventricular involvement. This algorithm worked well not only in their study, but could differentiate CS from ACM in the case of our patient [ 6 ] and on the ECG of the case report of Saturi G et al . [ 19 ], which are both case reports on patients with CS mimicking completely ACM (Fig.…”

“…R’ wave was defined as any positive deflection after an S wave. Reproduced with permission from [ 6 ]. ARVC, arrhythmogenic right ventricular cardiomyopathy; CS, cardiac sarcoidosis; ECG, electrocardiogram; ACM, arrhythmogenic cardiomyopathy.…”

“…While 90% of patients with sarcoidosis have lung and intrathoracic lymph node involvement, only approximately 5% have clinically manifest cardiac involvement. Another 20–25% of patients have asymptomatic, clinically silent cardiac involvement shown in autopsy studies [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 ]. Earlier studies [ 10 , 11 , 12 ] showed that most patients with clinically manifest cardiac sarcoidosis (CS) have minimal extracardiac disease and up to two thirds have isolated CS, however more recent studies [ 13 , 14 ] reported a much lower 3.2% to 9.4% prevalence of isolated CS without evidence of extracardiac disease using 18 F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT).…”

“…CS can be asymptomatic, subclinical or can present with conduction disturbances (atrioventricular block or intraventricular conduction disturbance), ventricular and atrial arrhythmias, heart failure or sudden cardiac death. Approximately 30% of 60-year-old patients presenting with unexplained high degree (Mobitz type II second degree or third degree) atrioventricular (AV) block or ventricular tachycardia (VT) are diagnosed with CS, therefore CS should be strongly considered in such patients [ 6 , 15 , 16 , 17 , 18 ]. CS is the second leading cause of death in patients with sarcoidosis after pulmonary sarcoidosis and the leading cause of death among Japanese sarcoidosis patients, therefore its early recognition is important, because its early treatment may prevent death from cardiovascular involvement [ 4 , 8 , 10 ].…”